title: Pulmonary lymphangioleiomyomatosis: a rare case.
abstract: Lymphangioleiomyomatosis is an uncommon lung disease primarily affecting women of childbearing age. It is characterized by the progressive proliferation and infiltration of smooth muscle-like cells, which lead to cystic destruction of the lung parenchyma; obstruction of airways, blood vessels, and lymphatics; and loss of pulmonary function. We present the case of a 46-year-old female patient with chest pain, cough, sputum, and dyspnea on exertion for three weeks. Minimal pneumothorax was noted, and the patient was referred to our center for further investigation and treatment. High-resolution computed tomography revealed numerous bilateral thin-walled air cysts and interstitial thickening affecting the central and peripheral part of the upper zone of the lung. We performed an open-lung biopsy to confirm lymphangioleiomyomatosis. Our aim is to discuss the pathogenesis and other lesions noted in the differential diagnosis of this rare disease.
authors.affiliation: Department of Pathology, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital , ANKARA, TURKEY.
authors.forename: Yetkin
authors.initials: Y
authors.lastname: Ağaçkiran
authors.forename: Arzu
authors.initials: A
authors.lastname: Ertürk
authors.forename: Fatma Irem
authors.initials: FI
authors.lastname: Yeşıller
authors.forename: Nevin Taci
authors.initials: NT
authors.lastname: Hoca
authors.forename: Leyla Nesrin
authors.initials: LN
authors.lastname: Ustün
authors.forename: Nermin
authors.initials: N
authors.lastname: Capan
mesh_terms: D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D008875:Middle Aged; D011030:Pneumothorax / Q000209:etiology